A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with osseous component is reported. A 23-year-old man was admitted to our hospital because of acute severe abdominal pain. Abdominal computed tomography revealed a large solid and cystic, oval shaped mass, measuring 11.0×6.0 cm in the pelvic cavity. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming Homer-Wright rosettes and perivascular pseudorosettes, and showed areas of osteoid and bone formation. Immunohistochemical studies revealed that tumor cells expressed positivity against CD99 (MIC2), CD57, neuron-specific enolase, and vimentin. Fluorescence in situ hybridization study revealed Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement on chromosome 22q12. To the authors' knowledge this is the first documentation of a peripheral neuroectodermal tumor with osteoid and bone formation of the small bowel mesentery.

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• Primary Ewing’s sarcoma in a small intestine – a case report and review of the literature
  Andrej Kolosov, Audrius Dulskas, Kastytis Pauza, Veslava Selichova, Dmitrij Seinin, Eugenijus Stratilatovas
  BMC Surgery.2020;[Epub]     CrossRef
• Case report and literature review of Ewing's sarcoma in the gastrointestinal tract
  Christopher Bong, Iain Thomson, Guy Lampe
  Surgical Practice.2018; 22(2): 84.     CrossRef
• Pediatric Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report
  You Sun Kim, Hye Min Moon, Kyu Sang Lee, Young Suk Park, Hyun-Young Kim, Ji Young Kim, Jin Min Cho, Hyoung Soo Choi
  Clinical Pediatric Hematology-Oncology.2017; 24(2): 162.     CrossRef
• Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature
  Zhe Liu, Yuan-Hong Xu, Chun-Lin Ge, Jin Long, Rui-Xia Du, Ke-Jian Guo
  World Journal of Clinical Cases.2016; 4(9): 306.     CrossRef
• Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of the literature
  LIBO PENG, LIMIN YANG, NAN WU, BO WU
  Experimental and Therapeutic Medicine.2015; 9(4): 1299.     CrossRef
• Une curieuse tumeur digestive à cellules rondes
  Alia Zehani, Ines Chelly, Beya Chelly, Jean-Michel Coindre, Slim Haouet, Nidhameddine Kchir
  Annales de Pathologie.2014; 34(2): 104.     CrossRef